Abstract 3093: Fatal Arrhythmias in Pediatric Left Ventricular Non-Compaction
Background: Left ventricular non-compaction (LVNC) is a recently recognized cardiomyopathy characterized by excessive trabeculation of the left ventricular myocardium, progressive myocardial dysfunction, and early mortality. LVNC has a heterogeneous clinical presentation that includes arrhythmia and sudden cardiac death.
Objectives: To examine the electrocardiographic and arrhythmic phenotype of children diagnosed with isolated LVNC.
Methods: A retrospective study was performed of all patients diagnosed with LVNC at a single academic pediatric hospital. Any patient with significant congenital heart disease was excluded. All electrocardiograms were interpreted by 2 blinded, independent pediatric electrophysiologists.
Results: Two hundred and forty two children were diagnosed with isolated LVNC over the study period utilizing standard diagnostic criteria. Thirty children died and 13 were transplanted. Twenty five children presented with arrhythmia, 5 after resuscitated sudden death. ECG abnormalities were present in 87% of patients. Ventricular hypertrophy and repolarization abnormalities were common ECG findings. Repolarization abnormalities were associated with increased mortality (p<0.05). Eighty patients had a documented arrhythmia. Patients with arrhythmia had increased mortality (p=0.002). Thirty six children had document ventricular tachycardia (VT). Seven children had VT in the first year of life with 5 of these dying in the first year of life (OR=12). There were 13 cases of sudden death with ventricular hypertrophy (p=0.02) and ST abnormalities (p=0.007) most strongly associated with arrhythmogenic death. The majority of patients with sudden death were noted to have cardiac dysfunction.
Conclusions: LVNC is strongly associated with life threatening arrhythmias in children. Children with arrhythmias and LVNC have increased mortality with ventricular arrhythmias in the first year of life carrying the worst prognosis. Children with LVNC should therefore have life long monitoring for ventricular arrhythmias.